S-Space College of Medicine/School of Medicine (의과대학/대학원) Dept. of Medicine (의학과) The Seoul Journal of Medicine The Seoul Journal of Medicine Vol. 34 No.3 (1993)
A Clinicopathologic Study of Infantile Hemangioendothelioma of the Liver
- Ha, Chang Won; Kim, Chong Jai; Chi, Je G.
- Issue Date
- Seoul J Med, Vol.34 No.3, pp. 181-186
- Infantile hemangioendothelioma which is the most common primary
vascular neoplasm of the liver in infancy and early childhood, frequently poses
diagnostic problems due to its characteristics, and thus the authors performed a
retrospective clinicopathological analysis of 5 cases of infantile hemangioendothelioma
of the liver. All the cases were found before 6 months of age, and
there were two females and three males. An abdominal mass and jaundice comprised
main symptoms, and associated cutaneous hemangiomas were not
present. A case had features of Kasabach-Merritt syndrome. The tumors were
well circumscribed, solid masses with areas of necrosis and hemorrhage, and
microscopically were composed of numerous vascular channels of varying size,
separated by fibrous stroma containing a single layer of flat or plump endothelial
cells which were located on the luminal side of the vascular reticulin sheath.
Immunohistochemical staining demonstrated factor VIII-related antigen in the
tumor cells, and ultrastructurally, relatively well formed vascular lumina were surrounded
by sparsely arranged elongated spindle cells having parallel processes.
Among the divergent primary hepatic neoplasms in infancy and childhood, this
tumor was found to have rather distinct clinicopathological features, and the
detailed differential points from other types of neoplasm were discussed along
with its anticipated biologic behaviour.